Treatment

The therapy of Sezary syndrome remains unsatisfactory. Patients may benefit from total body electron beam therapy, psoralen with UVA treatment (PUVA; alone or associated with etretinate, interferon-a2a, or both), or chlorambucil combined with prednisone (Winkelmann scheme) [1,4750]. Complete responses have been observed in several patients after extracorporeal photochemotherapy, but recurrence is the rule, and the efficacy has been debated [51-55]. An overall response rate of 62.5% (complete response 18.8%; partial response 43.7%) has been observed with pentostatin therapy in one study [56]. Patients with cutaneous tumours and/or large cell transformation should be treated with systemic chemotherapy or other aggressive treatment modalities.

Recently, new modalities have been introduced in the treatment of patients with Sezary syndrome, including pen-tostatin, IL-2, bone marrow transplantation, new retinoids such as bexarotene, and new chemotherapeutic agents (see Chapter 2) [57- 61]. The association of extracorporeal photo-pheresis with other treatments has also been tested in a limited number of patients [62]. No single treatment modality revealed clear-cut benefits in comparison to the others, and the management of these patients is still extremely problematic.

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